Pyoderma gangrenosum Preceding the diagnosis of systemic lupus erythematosus

Patients with systemic lupus erythematosus (SLE) often develop leg ulceration, particularly those with antiphospholipid antibodies or with vasculitis. Pyoderma gangrenosum (PG) is an idiopathic ulcerative neutrophilic dermatosis that is commonly associated with inflammatory bowel disease or seronegative polyarthritis. Although PG-like lesions have been commonly described in patients with the antiphospholipid antibody syndrome, the occurrence of PG as a preceding manifestation of SLE has only rarely been reported. We present a patient who developed PG roughly 8 years prior to developing SLE.     

Acquired ichthyosis and pyoderma gangrenosum in a patient with systemic lupus erythematosus

A 25-year-old woman who was 20 weeks pregnant developed systemic lupus erythematosus with acquired ichthyosis and pyoderma gangrenosum. The course of these dermatoses suggests that an immunopathological mechanism was implicated in their pathogenesis.     

Facial bullous systemic lupus erythematosus

A 37-year-old woman presented with a 6-month history of malaise, fever, arthralgia, and mild butterfly facial erythema. A diagnosis of systemic lupus erythematosus (SLE) was established, based on clinical, laboratory, and histologic findings, and treatment with 6-methylprednisolone (80 mg daily) was administered. A week later, a blistering eruption appeared in and around the erythematous areas on the face.
On physical examination, infiltrated erythematous plaques were present on the sun-exposed areas: the arms, dorsa of the hands, and upper chest. In and around the erythematous areas on the face, there were numerous tense vesicles (Fig. 1). A few ulcerations were observed on the lower lip and the hard palate.
Laboratory findings revealed a reduced white blood cell count (WBC) (2500/mm³), with normal values of hemoglobin, red blood cell count (RBC), hematocrit, platelets, creatlnine, urea, total protein, blood glucose, and urinalysis. Antinuclear antibodies (ANA) were detected in titre of 1:320 on rat liver substrate. Anti-DNA, Ro, La-antibodies and LE-cells were positive. Anti-BMZ-antibodies were not present on the indirect immunofluorescence (IIF) testing on normal human skin substrate. Histopathologic examination showed a subepidermal blister containing polymorphonuclears and eosinophils. There was a mixed Inflammatory infiltrate in the upper dermis (Fig. 2). Direct immunofluorescence (DIF) of perilesional skin from the face showed homogenous linear deposits of IgG and IgA at the basement membrane zone (BMZ); DiF from non-sun-exposed normal skin demonstrated granular deposits of IgA and IgM at the BMZ.
Besides the systemic steroid therapy with 6-methylprednisolone, a treatment with dapsone at an initial dosage of 50 mg, and later 100 mg, daily was applied. The condition was resistant to the steroid therapy, but after 4 weeks of treatment with dapsone the lesions completely resolved.     

Pyoderma gangrenosum with pathergy in a pregnant patient without associated systemic disease

Pyoderma gangrenosum (PG) is a rare chronic ulcerative skin condition often associated with systemic disease. PG associated with pregnancy is an extremely rare presentation; only 9 other cases have been reported in the literature. We present PG in a pregnant patient (third trimester) with pathergy. No associated systemic disease was identified. Histology was consistent with PG and the lesions responded to intralesional triamcinolone therapy.     

Association of bullous pemphigoid with systemic lupus erythematosus

Seven previous cases of coexistent bullous pemphigoid (BP) and systemic lupus erythematosus (SLE) have been reported. An 83-year-old man had clinical, laboratory, and histologic evidence of both BP and SLE. The question of whether coexistent SLE and BP actually occur can be settled only when complete studies are available.     

Pyoderma gangrenosum in a patient with essential thrombocythemia

BACKGROUND: Pyoderma gangrenosum is an uncommon ulcerative condition associated with inflammatory bowel disease, arthritis, and hematologic disease. We report a patient with essential thrombocythemia and pyoderma gangrenosum. OBJECTIVE: This article is a review of the associations between pyoderma gangrenosum and other diseases. RESULTS: There have been two previous reports of patients with pyoderma gangrenosum and essential thrombocythemia. CONCLUSION: There may be a possible association between pyoderma gangrenosum and essential thrombocythemia. The diagnosis of pyoderma gangrenosum should be considered in patients with essential thrombocythemia and cutaneous ulcers.     

大疱性系统性红斑狼疮临床资料分析

分析国内近20年发表的大疱性系统性红斑狼疮38篇文献,共57例,其中女47例,男10例,平均(26.6±11.8)岁;典型皮损为暗红色斑疹或正常皮肤上出现水疱大疱;常累及血液系统(64.9%)、肾脏(61.4%)及关节(57.9%);ANA阳性率为100%、dsDNA阳性率为66.7%、Sm抗体阳性率为59.6%;89.1%患者症状得到控制,其中23.6%患者单用糖皮质激素有效,76.4%患者联用DDS或其他免疫抑制剂。     

大疱性系统性红斑狼疮1例

报告1例大疱性系统性红斑狼疮，临床上符合SLE的诊断标准，并有大疱形成。组织病理改变为表皮下大疱形成，疱液中有许多嗜中性白细胞，Alcianblue染色阳性。     

大疱性系统性红斑狼疮3例及文献复习

报告3例大疱性系统性红斑狼疮(BsLE).3例患者均为女性,年龄分别为36、57和23岁.临床上均表现为全身泛发水疱、大疱.皮损组织病理检查显示为表皮下水疱,真皮乳头层较多中性粒细胞浸润,2例出现基底细胞液化变性.直接免疫荧光检查:2例示免疫球蛋白G(IgG)和(或)免疫球蛋白A(IgA)、免疫球蛋白M(IgM)抗体线状沉积于基膜带(BMZ),1例IgA线状沉积于BMZ,无lgG、IgM沉积.3例均符合SLE的诊断标准.糖皮质激素和氨苯砜可有效控制水疱、大疱的发生.单独以IgA介导的BSLE在临床上罕见.     

大疱性系统性红斑狼疮一例

<正>临床资料患者,女,18岁。主因口腔溃疡、双上肢水肿性或荨麻疹样红斑2个月,全身泛发大疱20余天,于2009年7月31日就诊。患者2个月前无明显诱因口腔出现多个血疱伴疼痛,疱壁破溃后形成溃疡,1周后双手背、上肢出现瘙痒性红斑、风团样皮损,双眼睑肿胀,结膜充血。在当地医院以荨麻疹给予咪唑斯汀等抗过敏药物治疗后皮损有所好转。1个月前,患者背部突然出现黄豆至核桃大小水疱,自觉瘙痒,     

Acquired haemophilia in a patient with systemic lupus erythematosus

INTRODUCTION: In patients with lupus, the most common acquired circulating anticoagulant is antiprothrombinase which is responsible for thrombosis. The presence of antibodies directed against factor VIII is rarely found in systemic lupus erythematosus. A case of acquired haemophilia in a patient with lupus is reported. CASE REPORT: A 30 year-old woman with systemic lupus erythematosus developed a right coxalgia and ecchymotic skin lesions which were prominent on the right arm and forearm. Laboratory values were as follows: positive antinuclear antibodies > 1: 2 560, anti-DNA antibodies (300 IU/ml), prolonged activated partial thromboplastin time, reduced factor VIII activity (1 p. 100) and the presence of antibodies against factor VIII. Magnetic nuclear resonance of the right hip confirmed the presence of an intramuscular hematoma. The patient was initially treated with intravenous pulse and oral corticosteroids, intravenous immunoglobulins and intravenous cyclophosphamide. Clinical and biological improvement was promptly obtained. DISCUSSION: In our patient with systemic lupus erythematosus, bleeding revealed acquired haemophilia with antibodies against factor VIII. It should be pointed out that the association between lupus and haemophilia is uncommon and that at present no standardized treatment can be recommended.     

Partial lipodystrophy in a patient with systemic lupus erythematosus

A 54-year-old woman developed partial lipodystrophy on the left side of her face. She had been suffering from systemic lupus erythematosus (SLE) since 1985 when she was 45 years old, and she had been treated with 30 mg/day of oral prednisolone as an initial dose. Partial lipodystrophy appeared on her left lower jaw in 1994 when the SLE was inactive, and the dose of prednisolone was reduced to 5 mg/ 3 days. Gradually, the lipodystrophy spread toward her left cheek and her left forehead without any preceding skin symptoms. Histological examination showed a loss of fat tissue and mild lymphocytic infiltrations mainly around cutaneous appendages and vessels in the dermis and subcutaneous tissue. The dose of prednisolone was increased to 10 mg/day and the lesions stopped spreading. Such partial lipodystrophy is distinct from lipoatrophy of lupus profundus.     

Pyoderma gangrenosum in HIV patient

Pyoderma gangrenosum (PG) has previously been reported in very few patients with HIV infection. We describe here a 30-year-old housewife with HIV infection having multiple ulcerations over lower limbs characteristic of PG.     

Coexistent bullous pemphigoid and systemic lupus erythematosus.

A case of coexistent bullous pemphigoid and systemic lupus erythematosus is reported. Evidence for an immunologic pathogenesis is reviewed for both disorders. It is suggested that the coexistence of bullous pemphigoid and systemic lupus erythematosus may represent a similar pathomechanism.